Acromegaly resident survival guide: Difference between revisions

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*** [[Hypothalamic hamartoma]], [[choristoma]], [[ganglioneuroma]]
*** [[Hypothalamic hamartoma]], [[choristoma]], [[ganglioneuroma]]
** Peripheral ectopic (1 percent)
** Peripheral ectopic (1 percent)
*** [[Carcinoid tumor|Bronchial carcinoid]], [[Pancreatic cancer|pancreatic islet-cell tumor]], [[small cell lung cancer]], [[adrenal adenoma]], [[medullary thyroid carcinoma]], [[pheochromocytoma]] <ref name="pmid12015856">{{cite journal| author=Altstadt TJ, Azzarelli B, Bevering C, Edmondson J, Nelson PB| title=Acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report. | journal=Neurosurgery | year= 2002 | volume= 50 | issue= 6 | pages= 1356-9; discussion 1360 | pmid=12015856 | doi=10.1097/00006123-200206000-00029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12015856  }} </ref><ref name="pmid10861322">{{cite journal| author=Beuschlein F, Strasburger CJ, Siegerstetter V, Moradpour D, Lichter P, Bidlingmaier M | display-authors=etal| title=Acromegaly caused by secretion of growth hormone by a non-Hodgkin's lymphoma. | journal=N Engl J Med | year= 2000 | volume= 342 | issue= 25 | pages= 1871-6 | pmid=10861322 | doi=10.1056/NEJM200006223422504 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10861322  }} </ref>
*** [[Carcinoid tumor|Bronchial carcinoid]], [[Pancreatic cancer|pancreatic islet-cell tumor]], [[small cell lung cancer]], [[adrenal adenoma]], [[medullary thyroid carcinoma]], [[pheochromocytoma]] <ref name="pmid12015856">{{cite journal| author=Altstadt TJ, Azzarelli B, Bevering C, Edmondson J, Nelson PB| title=Acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report. | journal=Neurosurgery | year= 2002 | volume= 50 | issue= 6 | pages= 1356-9; discussion 1360 | pmid=12015856 | doi=10.1097/00006123-200206000-00029 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12015856  }} </ref><ref name="pmid10861322">{{cite journal| author=Beuschlein F, Strasburger CJ, Siegerstetter V, Moradpour D, Lichter P, Bidlingmaier M | display-authors=etal| title=Acromegaly caused by secretion of growth hormone by a non-Hodgkin's lymphoma. | journal=N Engl J Med | year= 2000 | volume= 342 | issue= 25 | pages= 1871-6 | pmid=10861322 | doi=10.1056/NEJM200006223422504 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10861322 }} </ref><ref name="pmid32595044">{{cite journal| author=| title=Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5. | journal=Lancet Infect Dis | year= 2020 | volume= 20 | issue= 7 | pages= e148 | pmid=32595044 | doi=10.1016/S1473-3099(20)30370-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=32595044 }} </ref>


==Diagnosis==
==Diagnosis==

Revision as of 15:55, 20 August 2020

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Tayyaba Ali, M.D.[2]

Overview

This section provides a short and straight to the point overview of the disease or symptom. The first sentence of the overview must contain the name of the disease.

Causes

Life Threatening Causes

Life-threatening causes include conditions that may result in death or permanent disability within 24 hours if left untreated. There is no life-threatening cause of acromegaly. However, if left untreated, 30% of patients with acromegaly may progress to develop cardiovascular manifestations, pulmonary dysfunction, and cerebral complications. These comorbidities will increase the mortality rate. [1]

Common Causes

Diagnosis

The approach to diagnosis of Acromegaly is based on a step-wise testing strategy. Below is an algorithm summarising the identification and laboratory diagnosis of Acromegaly.[9]

 
 
 
 
 
 
 
 
 
 
 
 
Characterize the symptoms:

Headaches
❑ Enlargement of the hands (change in ring or glove size) and feet (change in shoe size)
Lethargy
Hyperhidrosis (excessive sweating)
Paraesthesia [10]
Fatigue
Jaw pain
❑ Body odor
Blood in the stool
Sleep apnea
Weight gain [11]

❑ In males:
Sexual dysfunction
Loss of libido
Gynecomastia [12]

❑ In females:
Amenorrhea
Galactorrhea [12]
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Examine the patient:

❑ HEENT

❑ Musculoskeletal exam:

❑ Neurological exam:

❑ Cardiovascular exam:

❑ Skin exam:

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Measure Insulin like growth factor-1 (IGF-1) levels
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal
 
Equivocal
 
Elevated
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Active acromegaly ruled out
 
Oral glucose tolerance test (OGTT) with growth hormone (GH) levels
 
Acromegaly confirmed in a patient with typical clinical manifestations
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Growth hormone (GH) suppressed
 
 
 
 
Inadequate suppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Active acromegaly ruled out
 
 
 
 
 
Order pituitary MRI
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal
 
Mass or empty sella
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Chest and abdominal CT, Growth hormone-releasing hormone (GHRH) measurement
 
GH-secreting pituitary adenoma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Extra-pituitary acromegaly
 


This algorithm developed and modified according to Endocrine Society (ES): Clinical practice guideline on acromegaly.

Treatment

Shown below is an algorithm summarizing the treatment of Acromegaly according the the Endocrine Society (ES): Clinical practice guideline on acromegaly.

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Transphenoidal surgery
❑ Complete resection
❑ Tumors that are unresectable, a surgical debulking procedure may be performed followed by medical therapy
 
Yes
 
Patient is not a surgical candidate
❑ Patient preference
❑ High risk due to medical comorbidities
❑ Unresectable tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Are the following criteria met postoperatively?
❑ Morning serum GH the day after surgery <1ng/ml
❑ 12 weeks postoperative:
  • Normal serum IGF-1 (for age and gender)
  • No evidence of residual tumor on pituitary MRI
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Yes
 
No
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Remission
❑ Monitor with annual IGF-1
 
Is there residual tumor that appears resectable and readily accessible (eg, not invading the cavernous sinus)?
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Perform MRI for clinical or biochemical evidence of recurrence
 
Medical therapy

Somatostatin analogs:

  • Preferred regimen (1): Octreotide 50 mcg q8hr subcutaneous as initial dose and 100 mcg q8hr as effective dose.
  • Preferred regimen (2): Lanreotide 90 mg q4week for 3 months every 4 weeks subcutaneous.
  • Preferred regimen (3): Pasireotide 40 mg q4wk intramuscular.

Dopamine agonists:

  • Preferred regimen (1): Cabergoline 0.25 mg 2x/week orally.
  • Preferred regimen (2): Bromocriptine 1.25-2.5 mg qDay orally.

GH receptor antagonist:

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Failure of medical therapy
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Radiation therapy
❑ Stereotactic radiotherapy is most common method
 
 
 
 
 


This algorithm developed and modified according to Endocrine Society (ES): Clinical practice guideline on acromegaly.

Do's

  • The content in this section is in bullet points.

Don'ts

  • The content in this section is in bullet points.

References

  1. Melmed S (2009). "Acromegaly pathogenesis and treatment". J Clin Invest. 119 (11): 3189–202. doi:10.1172/JCI39375. PMC 2769196. PMID 19884662.
  2. Landis CA, Masters SB, Spada A, Pace AM, Bourne HR, Vallar L (1989). "GTPase inhibiting mutations activate the alpha chain of Gs and stimulate adenylyl cyclase in human pituitary tumours". Nature. 340 (6236): 692–6. doi:10.1038/340692a0. PMID 2549426.
  3. Vallar L, Spada A, Giannattasio G (1987). "Altered Gs and adenylate cyclase activity in human GH-secreting pituitary adenomas". Nature. 330 (6148): 566–8. doi:10.1038/330566a0. PMID 2825031.
  4. Hayward BE, Barlier A, Korbonits M, Grossman AB, Jacquet P, Enjalbert A; et al. (2001). "Imprinting of the G(s)alpha gene GNAS1 in the pathogenesis of acromegaly". J Clin Invest. 107 (6): R31–6. doi:10.1172/JCI11887. PMC 208949. PMID 11254676.
  5. Melmed S, Ezrin C, Kovacs K, Goodman RS, Frohman LA (1985). "Acromegaly due to secretion of growth hormone by an ectopic pancreatic islet-cell tumor". N Engl J Med. 312 (1): 9–17. doi:10.1056/NEJM198501033120103. PMID 2981107.
  6. Altstadt TJ, Azzarelli B, Bevering C, Edmondson J, Nelson PB (2002). "Acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report". Neurosurgery. 50 (6): 1356–9, discussion 1360. doi:10.1097/00006123-200206000-00029. PMID 12015856.
  7. Beuschlein F, Strasburger CJ, Siegerstetter V, Moradpour D, Lichter P, Bidlingmaier M; et al. (2000). "Acromegaly caused by secretion of growth hormone by a non-Hodgkin's lymphoma". N Engl J Med. 342 (25): 1871–6. doi:10.1056/NEJM200006223422504. PMID 10861322.
  8. "Correction to Lancet Infectious Diseases 2020; published online April 29. https://doi.org/10.1016/ S1473-3099(20)30064-5". Lancet Infect Dis. 20 (7): e148. 2020. doi:10.1016/S1473-3099(20)30370-4. PMID 32595044 Check |pmid= value (help). External link in |title= (help)
  9. 9.0 9.1 Katznelson L, Laws ER, Melmed S, Molitch ME, Murad MH, Utz A; et al. (2014). "Acromegaly: an endocrine society clinical practice guideline". J Clin Endocrinol Metab. 99 (11): 3933–51. doi:10.1210/jc.2014-2700. PMID 25356808.
  10. 10.0 10.1 10.2 Molitch ME (1992). "Clinical manifestations of acromegaly". Endocrinol Metab Clin North Am. 21 (3): 597–614. PMID 1521514.
  11. 11.0 11.1 11.2 "Acromegaly: MedlinePlus Medical Encyclopedia".
  12. 12.0 12.1 Iuliano SL, Laws ER (2014). "Recognizing the clinical manifestations of acromegaly: case studies". J Am Assoc Nurse Pract. 26 (3): 136–42. doi:10.1002/2327-6924.12076. PMID 24170330.
  13. Ben-Shlomo A, Melmed S (2006). "Skin manifestations in acromegaly". Clin Dermatol. 24 (4): 256–9. doi:10.1016/j.clindermatol.2006.04.011. PMID 16828406.


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